A case of low-grade endometrial stromal sarcoma (LGESS) with striking involvement of pelvic veins is reported. The pattern of vascular involvement resembled that seen in intravenous leiomyomatosis. The report illustrated that the possibility of venous involvement should always be considered in the management of LGESS. Search for tumor involvement of the venous system at the time of diagnosis of metastasis or recurrence is essential. Surgical management of extensive venous recurrence of LGESS is feasible.